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AimTo present a rare case of extradigital glomus tumor (GT) located in the hypothenar region of the hand and to highlight the diagnostic challenges and clinical importance of considering GT in persistent nodular lesions of the upper extremity.CaseA 59-year-old female presented with a one-year history of swelling and tenderness in the hypothenar region of her right hand that was unresponsive to medical treatment. Physical examination revealed a palpable subcutaneous nodular lesion with marked tenderness, without skin discoloration or cold intolerance. The Hildreth test was positive. Plain radiographs demonstrated no bony pathology. Doppler ultrasonography showed a hypervascular hypoechoic mass, while magnetic resonance imaging revealed a lesion hypointense on T1-weighted and hyperintense on T2-weighted sequences. The lesion was surgically excised under regional anesthesia with tourniquet control. Histopathological and immunohistochemical findings were consistent with a GT. The patient’s symptoms resolved completely following surgery, and no recurrence was observed during follow-up.ConclusionExtradigital GTs may lack classic clinical features such as discoloration or cold sensitivity, which can delay diagnosis. Persistent pain unresponsive to conservative treatment should raise suspicion. GTs should be considered in the differential diagnosis of both painful and painless nodular lesions of the upper extremities, and complete surgical excision remains the treatment of choice to achieve symptom relief and prevent recurrence. Keywords: glomus tumor; hand; hypothenar region; pain